Meconium ileus is an early manifestation of cystic fibrosis (CF), due to abnormal, inspissated, and viscid mucus of intestinal origin. In children affected by this condition, the impacted meconium produces an intraluminal obstruction occurring in the midileum, leading to a progressive distension. As an ultimate evolution, different mechanical complications can be associated, including intestinal volvulus, atresia, gangrene and necrosis, perforation, peritonitis with abdominal calcifications, and finally, meconial pseudocyst. In this view, meconium diseases in infancy cannot be firmly separated into three categories, such as meconium plug syndrome, meconium ileus, and meconium peritonitis, nor can therapy of each condition.